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Pulmonology›Condition·Updated Jun 27, 2026·v1

Pulmonary Hypertension

Pulmonary hypertension is defined hemodynamically by mPAP >20 mmHg and comprises five WHO groups with distinct pathophysiologies. Diagnosis requires RHC with PAWP and PVR. Group 1 PAH is treated with upfront ERA+PDE5i combination, escalated to parenteral prostacyclin or sotatercept in high-risk patients. Group 4 CTEPH is potentially curable with PEA; inoperable cases use riociguat. The RV is the central determinant of outcomes; RV-PA uncoupling (Ees/Ea <0.8) identifies patients at highest risk. Key therapeutic advances include sotatercept (activin signaling inhibition) and the AMBITION regimen of ambrisentan + tadalafil.

High Evidence124 references·6,178 words·25 min read·v1
pulmonary hypertensionpulmonary arterial hypertensionright heart catheterizationprostacyclinsotaterceptCTEPHRV failure

Quick Reference

RxDrug of choiceEpoprostenol IV continuous infusion (2 ng/kg/min initial, titrate) for high-risk PAH; ambrisentan 10 mg + tadalafil 40 mg daily for low-risk (AMBITION regimen). For CTEPH: riociguat 2.5 mg TID.
AltAlternativesMacitentan 10 mg daily, sildenafil 20 mg TID, selexipag 200 mcg BID (oral), iloprost inhaled, sotatercept 0.3-0.7 mg/kg SC q3weeks
⊘AvoidPDE5 inhibitors with nitrates or riociguat; non-dihydropyridine CCBs (diltiazem, verapamil) in PAH; PAH-specific drugs in Group 2 or 3 PH (can worsen hemodynamics)
DxTest of choiceRight heart catheterization (RHC) to confirm mPAP >20 mmHg and classify pre- vs post-capillary (PAWP ≤15 vs >15 mmHg)
ScKey scoreWHO functional class (I-IV); 6-minute walk distance (<165 m high risk); NT-proBNP (<300, 300-1400, ≥1400 ng/L); REVEAL 2.0 risk score (incorporated in 2022 ESC/ERS guidelines)
→When to referAll new PH diagnoses should be referred to a PH center; urgent referral for WHO FC III/IV, syncope, acute RV failure, or CTEPH for PEA evaluation
PH requires RHC for diagnosis; treat based on WHO group, never use PAH drugs in Group 2. For Group 1, start upfront combination (ambrisentan + tadalafil); escalate to parenteral prostacyclin if high risk. CTEPH is potentially curable with PEA. The RV is the key prognostic organ, monitor coupling (Ees/Ea).
Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure (mPAP) >20 mmHg at rest, affecting ~1% of the global population and rising to >10% in those over 65. It is a hemodynamic syndrome, not a single disease, encompassing five WHO groups. The most critical diagnostic step is right heart catheterization (RHC) to confirm PH and distinguish pre-capillary (Group 1, 4) from post-capillary (Group 2) disease, as this entirely alters management. PH is the third leading cause of cardiovascular death, with untreated Group 1 PAH carrying a 5-year survival of only 28% in advanced stages.

Overview and Recommendations

Background

  • •Pulmonary hypertension (PH) is a hemodynamic syndrome defined by a mean pulmonary artery pressure (mPAP) >20 mmHg, a threshold lowered in 2018 from >25 mmHg to reflect that normal mPAP is 14±3 mmHg and values 20-25 mmHg carry elevated risk. The syndrome affects ~1% of the global population, rising to >10% in those over age 65, driven largely by WHO Group 2 PH due to heart failure with preserved ejection fraction (HFpEF). PH is the third leading cause of cardiovascular death, with a 5-year survival of only 28% in WHO functional class IV despite modern therapy.
  • •The five WHO clinical groups capture divergent etiologies: Group 1 (pulmonary arterial hypertension, PAH) includes idiopathic, heritable (BMPR2 mutations in ~70%), drug-induced, connective tissue disease-associated, HIV, and portopulmonary hypertension; Group 2 (PH due to left heart disease) is the most common form; Group 3 (PH due to lung diseases/hypoxia) includes COPD and interstitial lung disease; Group 4 (chronic thromboembolic PH, CTEPH) is potentially curable with pulmonary endarterectomy; and Group 5 encompasses multifactorial mechanisms like sarcoidosis and metabolic disorders.
  • •The central pathophysiology in PAH (Group 1) is a cancer-like phenotype in pulmonary artery smooth muscle cells (PASMCs): they are proliferative, apoptosis-resistant, and metabolically reprogrammed toward glycolysis (Warburg effect) via suppressed Kv1.5 potassium channels and mitochondrial fragmentation. Endothelial dysfunction shifts the balance from vasodilation (NO/cGMP, prostacyclin) to vasoconstriction (endothelin-1). In IPAH, a landmark trial PATENT-1 (2013) showed riociguat improved 6-minute walk distance by +36 m; SERAPHIN (2013) demonstrated macitentan reduced morbidity/mortality (HR 0.55).
  • •Right ventricular (RV) adaptation determines clinical outcomes. The RV is afterload-sensitive; when mPAP exceeds ~40 mmHg, the RV transitions from compensatory hypertrophy to dilatation and failure (RV-PA uncoupling, Ees/Ea <0.8). The gold-standard measure of RV coupling is the Ees/Ea ratio, with a ratio <0.8 predicting clinical worsening and mortality. Speckle-tracking echocardiography provides a non-invasive surrogate via RV free-wall longitudinal strain (RV-FWS).

Evaluation

  • •Suspect PH in any patient with unexplained exertional dyspnea, fatigue, presyncope/syncope, or chest pain, especially if there is a history of connective tissue disease, HIV, portal hypertension, congenital heart disease, or a family history of PH.
  • •Ask about the timing and progression of dyspnea (insidious over months to years), orthopnea or paroxysmal nocturnal dyspnea (suggesting left heart disease, Group 2), prior pulmonary embolism or deep vein thrombosis (Group 4), sleep apnea or snoring (Group 3), and risk factors for HIV, drug/toxin use (anorexigens, methamphetamine), or Raynaud phenomenon (connective tissue disease).
  • •Examine for signs of right heart strain: elevated jugular venous pressure (JVP) with prominent a and v waves, a right ventricular heave at the left parasternal border, a loud P2 (pulmonary component of S2), and a holosystolic tricuspid regurgitation murmur that increases with inspiration. Also look for signs of RV failure: hepatomegaly, ascites, and peripheral edema.
  • •Examine for clues to underlying etiology: crackles suggest left heart disease (Group 2); digital clubbing or hyperresonance suggest lung disease (Group 3); telangiectasias or sclerodactyly suggest systemic sclerosis (Group 1). Perform a full cardiopulmonary exam including oxygen saturation at rest and with ambulation.
  • •Order transthoracic echocardiogram (TTE) as the first-line screening test. Estimate systolic pulmonary artery pressure (sPAP) from the tricuspid regurgitation jet velocity (4v² + right atrial pressure). High probability of PH is sPAP >45 mmHg or RV dilation/dysfunction. Normal TTE does not exclude PH, obtain RHC if clinical suspicion remains high.
  • •If TTE suggests PH, order right heart catheterization (RHC) for definitive diagnosis. RHC confirms mPAP >20 mmHg, measures pulmonary artery wedge pressure (PAWP) and cardiac output (by thermodilution or Fick), and calculates PVR = (mPAP - PAWP)/CO. Pre-capillary PH requires PAWP ≤15 mmHg and PVR >2 Wood units.
  • •During RHC, perform acute vasoreactivity testing with inhaled nitric oxide or epoprostenol in selected patients with IPAH (not in others). A positive response (mPAP drop >10 mmHg to ≤40 mmHg with preserved cardiac output) identifies candidates for high-dose calcium channel blocker therapy (~10% of IPAH patients).
  • •Use the diagnostic criteria from the 2022 ESC/ERS guidelines: the diagnosis of PH requires RHC with mPAP >20 mmHg. Group 1 (PAH) requires pre-capillary hemodynamics (PAWP ≤15, PVR >2 WU) and exclusion of other groups. Group 2 (PH-LHD) requires PAWP >15. Group 4 (CTEPH) requires mismatched perfusion defects on V/Q scan after ≥3 months of anticoagulation.
  • •Also consider: cardiac MRI for RV volumetric and functional assessment (gold standard for RVEF); pulmonary function tests with DLCO; high-resolution CT chest to exclude interstitial lung disease; V/Q scan to rule out CTEPH; and polysomnography if sleep apnea is suspected. Check NT-proBNP for baseline risk stratification (<300 ng/L low risk, ≥1400 ng/L high risk).
  • •Assess functional status using the WHO functional class (I-IV) and 6-minute walk distance. WHO FC II predicts better prognosis than III/IV. A 6-minute walk distance <165 m or desaturation to <88% during walk identifies high-risk patients.

Management

  • •Initiate therapy based on WHO group, never use PAH-specific therapies (endothelin receptor antagonists, PDE5 inhibitors, prostacyclin analogs) in Group 2 (PH-LHD) as they can worsen hemodynamics. For Group 4 CTEPH, refer for pulmonary endarterectomy (PEA) if anatomically suitable; inoperable CTEPH is treated with riociguat (2.5 mg TID, titrated from 1 mg TID).
  • •For Group 1 PAH, start combination therapy upfront in low- or intermediate-risk patients: ambrisentan 10 mg daily + tadalafil 40 mg daily (from AMBITION trial, 2015) reduced clinical failure by 50% vs monotherapy. For high-risk patients, initiate triple therapy including a parenteral prostacyclin.
  • •Administer endothelin receptor antagonists (ERAs): ambrisentan 5-10 mg daily; macitentan 10 mg daily (SERAPHIN trial, risk reduction HR 0.55); bosentan 62.5-125 mg BID, requires monthly LFT monitoring due to hepatotoxicity. ERAs are teratogenic, ensure negative pregnancy test monthly.
  • •Administer PDE5 inhibitors: sildenafil 20 mg TID (STARTS-1/2), but higher doses (40 mg TID) are associated with increased mortality; tadalafil 40 mg daily (PHIRST trial, +33 m 6MWD). Both are contraindicated with nitrates or riociguat due to risk of severe hypotension.
  • •Administer prostacyclin pathway agents: epoprostenol IV continuous infusion (start 2 ng/kg/min, titrate up by 1-2 ng/kg/min every 15 min as tolerated) is first-line for high-risk PAH; treprostinil SC/IV/inhaled; iloprost inhaled (2.5-5 mcg 6-9x daily). All cause infusion site pain, headache, flushing, jaw pain, and thrombocytopenia. Selexipag (oral IP receptor agonist, GRIPHON trial) reduces morbidity/mortality (HR 0.60).
  • •Administer riociguat (sGC stimulator) 1 mg TID, titrate to 2.5 mg TID as tolerated (PATENT-1, +36 m 6MWD, improved hemodynamics). Contraindicated with PDE5 inhibitors due to hypotension risk. Use only for PAH and CTEPH.
  • •For new approval: sotatercept (activin signaling inhibitor) 0.3-0.7 mg/kg SC every 3 weeks, targets vascular remodeling. In STELLAR trial (2023), it reduced clinical worsening (HR 0.52) and improved 6MWD (+33 m). Side effects include telangiectasia, GI bleeding, and thrombocytopenia. Reserved for advanced PAH in combination with other therapies.
  • •Titrate to target doses: ambrisentan 10 mg daily, macitentan 10 mg daily, sildenafil 20 mg TID, tadalafil 40 mg daily. For epoprostenol, target dose is typically 30-50 ng/kg/min. Escalate therapy if WHO FC deteriorates or 6-minute walk distance declines >10%. Reassess risk at 3-6 months: if intermediate or high risk, escalate to triple therapy.
  • •Monitor with echocardiogram every 6-12 months and every 3 months in high-risk patients. NT-proBNP every 3 months; 6-minute walk test at each visit. Check LFTs monthly before ERAs. For CTEPH post-PEA, repeat RHC at 3-6 months to assess residual PH.
  • •What NOT to do: avoid non-dihydropyridine CCBs (diltiazem, verapamil) in PAH, they reduce contractility and can worsen RV function. Do not use PAH-specific therapies in Group 2 or 3 PH. Do not use high-dose sildenafil (40 mg TID) due to mortality signal.
  • •Refer to a pulmonary hypertension center for all newly diagnosed PH, especially for RHC interpretation, vasoreactivity testing, and consideration of lung transplantation. Refer for PEA if CTEPH is anatomically suitable. Refer for lung or heart-lung transplantation if WHO FC III/IV despite maximal therapy.
  • •For acute decompensated RV failure: admit to ICU, reduce RV afterload with parenteral prostacyclin (epoprostenol), consider inotropes (dobutamine 2-20 mcg/kg/min, milrinone 0.125-0.75 mcg/kg/min), diurese for volume overload (furosemide IV 40-80 mg, titrate to urine output), and avoid positive pressure ventilation if possible (reduces preload). Discharge criteria include stable hemodynamics, improving RV function, and ability to tolerate oral therapy.

Board Review — High Yield

  • •mPAP >20 mmHg, new 2018 threshold; down from >25 mmHg, based on normal 14±3 mmHg
  • •RHC is mandatory, echocardiogram estimates probability but cannot confirm PH or group
  • •WHO Group 2, most common PH; due to left heart disease (HFpEF, valvular); PAWP >15 mmHg
  • •Estrogen paradox, women have 3-4× higher PAH incidence but better RV function and survival
  • •BMPR2 mutation, found in ~70% of heritable PAH; causes PASMC proliferation and apoptosis resistance
  • •Sotatercept, activin signaling inhibitor; reduces clinical worsening (HR 0.52) and improves 6MWD (+33 m)
  • •CTEPH, occurs in 3-5% after acute PE; treat with PEA; inoperable cases use riociguat
  • •Kv1.5 suppression, depolarizes PASMC, inhibits apoptosis, promotes contraction, key in PAH
  • •Ees/Ea <0.8, RV-PA uncoupling; predicts clinical worsening and mortality
  • •10% radius reduction → 46% PVR increase, fourth-power relationship; explains severity of early remodeling

Deep Dive — Evidence Details

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